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Hematology

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… . ↑ INR, ↑ PTT, ↓ fibrinogen (although it can be normal or even elevated), ↓ factor VIII (in contrast to liver diseases, which have normal factor VIII). Schistocytes on peripheral smear treatments — treat underlying cause and complications (hypoxia, dehydration, acidosis, acute renal failure). … Vitamin K deficiency ( vitamin K 10 mg PO/SC daily ×3 days). vWD type I ( DDAVP 0.3 μg/kg SC, intermediate purity factor VIII) Specific Entities Von Willebrand Disease (VWD) pathophysiology —vWF acts as a linker between platelets and endothelium and also serves as carrier for factor VIII. …

Bleeding Propensity in Waldenström Macroglobulinemia: Potential Causes and Evaluation.

Waldenström macroglobulinemia (WM) is a rare, incurable, low-grade, B cell lymphoma. Symptomatic disease commonly results from marrow or organ infiltration and hyperviscosity secondary to immunoglobulin M paraprotein, manifesting as anemia, bleeding and neurological symptoms among others. The causes of the bleeding phenotype in WM are complex and involve several intersecting mechanisms. Evidence of defects in platelet function is lacking in the literature, but factors impacting platelet function and coagulation pathways such as acquired von Willebrand factor syndrome, hyperviscosity, abnormal

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… VIII inhibitory activity Ann Intern Med 1972 77 04 571 575 4118604 10.7326/0003-4819-77-4-571 50 Castaldi P A Penny R A macroglobulin with inhibitory activity against coagulation factor VIII Blood 1970 35 03 370 376 4985542 51 Mazurier C Parquet-Gernez A Descamps J Bauters F Goudemand M Acquired von … VIII J Thromb Haemost 2014 12 01 36 42 24118899 10.1111/jth.12412 137 Federici A B The factor VIII/von Willebrand factor complex: basic and clinical issues Haematologica 2003 88 06 EREP02 12826528 138 Saraya A K Kasturi J Kishan R A study of haemostasis in macroglobulinaemia Acta Haematol 1972 47 …

Abstract

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… On the 8‐th day of hospitalization NovoSeven was stopped as factor VIII level increased to 6.2% and the inhibitors dropped to 1.1BU. … City of Health and Science, Turin, Turin, Piemonte, Italy Background : Acquired haemophilia A (AHA) is a severe bleeding disorder caused by the development of antibodies (inhibitors) to factor VIII (FVIII). Recombinant porcine factor VIII (rpFVIII, susoctocog alfa, Obizur®, Baxalta US Inc. …

Артериальный тромбоэмболизм

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Abstract

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MPO-ANCA-Associated Hypertrophic Pachymeningitis Mimicking IgG4-Related Disease: A Case Report and Literature Review.

Hypertrophic pachymeningitis (HP) is a rare and chronic clinical disease characterized by thickening of the dura mater, leading to persistent headache, cranial neuropathy, seizures, and other neurological symptoms. Immune-mediated causes, particularly antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and IgG4-related disease (IgG4-RD), are among the most common etiologies. We report a case of a 54-year-old female with recurrent headache, blepharoptosis, hearing loss, and markedly elevated inflammatory markers. Blood tests, and serum levels of IgG4 were within normal ranges. Cont

Delayed gastric emptying in critical illness: is enhanced enterogastric inhibition with cholecystokinin and peptide YY involved?

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… For this reason, commercial factor VIII concentrates, recombinant or treated to inactivate viruses, are preferred over cryoprecipitate for treating hemophilia A patients. … This new agent was created for the treatment of hemophiliac patients with high titers of antibodies to factor VIII, which makes them unable to benefit from transfusion of recombinant factor VIII. …

Shilajit mitigates chemotherapeutic drug-induced testicular toxicity: Study on testicular germ cell dynamics, steroidogenesis modulation, and Nrf-2/Keap-1 signaling.

BACKGROUND: Medications, including chemotherapeutic drugs, contribute to male infertility as external factors by inducing oxidative stress in testicular cells. Shilajit is a naturally occurring bioactive antioxidant used in Ayurvedic medicine to treat a variety of ailments. OBJECTIVE: This study examines the potential of Shilajit to counteract the negative effects of the chemotherapeutic drug cyclophosphamide (CPA) on testicular germ cell dynamics. MATERIAL AND METHODS: Male Parkes mice received single intraperitoneal CPA injection (200 mg/kg BW) on day one, followed by daily supplementation

A rare case of oral multisystem Langerhans cell histiocytosis.

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by high proliferation of Langerhans dendritic cells. LCH is a solitary or multifocal disease that primarily involves bone tissue and often affects children and young men. A 29 years-old Caucasian man was referred to the Oral Surgery Unit of George Eastman Hospital - Umberto I teaching hospital, with third degree mobility of teeth belonging to second, third and fourth quadrant. Panoramic radiograph showed multiple radiolucent areas with well demarcated borders on the right and left site of the mandible and on the left site of

Исследование по вопросу о факторах риска и стратегиях лечения глубокого веинского тромбоза у пациентов с множественной миеломой.

Множественная миелома (ММ) — злокачественная опухоль, появляющаяся из плазменных клеток, и в последние годы в нашей стране наблюдается значительная тенденция к росту ее распространения. Венозный тромбоэмболизм (VTE) является относительно распространенным осложнением у нескольких пациентов с миэломой (MM). Венозный тромбоэмболизм включает в себя тромбоз глубокой вены (ДВТ) и легочную эмболию (ПЕ), при этом во время лечения пациенты подвергаются высокому риску ДВТ. Исследования показали, что прокоагуляционное состояние MM, подстрекательская реакция и использованные терапевтические препараты знач

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… VIII, von Willebrand factor, antiphospholipid antibodies, and d-dimer levels, all of which can contribute to the occurrence of DVT. 17–20 Inflammatory Cells The release of inflammatory cytokines, especially interleukins, may change the coagulation mechanism to a hypercoagulable state, 21 with chronic … Extremely high levels of von Willebrand factor antigen and of procoagulant factor VIII found in multiple myeloma patients are associated with activity status but not with thalidomide treatment. J Thromb Haemost . 2003;1(3):445–449. doi: 10.1046/j.1538-7836.2003.00083.x 33. Palumbo A, Palladino C. …

The concept of multidimensional frailty in geriatric oncology: a systematic scoping review.

BACKGROUND: One of the major challenges in geriatric oncology is to estimate the risk-benefit balance of any therapeutic proposal for each patient and frailty is one of the key components of Comprehensive Geriatric Assessment (CGA)and can also be assessed using stand-alone screening tools that predicts functional decline, treatment-related complications and mortality. This study aimed to provide an overview of the existing literature on the concept of multidimensional frailty in older adults with cancer, to clarify the dimensions included in multidimensional frailty, and the components of frai

Диагностика множественных склерозов: из материалов Конференции Национального общества по проблемам множественных склерозов.

28-летняя чернокожая женщина имела как типичные, так и нетипичные особенности множественного склероза в условиях многоженства, включая психиатрическую историю, осложняющие диагностику и лечение. Этот случай иллюстрирует важность дифференциального диагноза и продольного контроля, прежде чем брать на себя обязательство по профилактике заболеваний. Особое внимание уделяется принятию решений в отношении индивидуального лечения.

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… Case Presentation In June 2023, a 28-year-old Black woman with a four-year history of unspecified mood disorder, migraine without aura, type 2 diabetes, obesity, hereditary factor VIII deficiency with recurrent unprovoked deep venous thrombosis (DVT)/pulmonary embolism (PE), and hidradenitis suppurativa … VIII elevation, 4 which may have predisposed her to cerebral venous thrombosis or DVT accompanied by paradoxical embolism causing ischemic stroke. …

Acquired von Willebrand syndrome and lymphoproliferative disorders: A case report.

Acquired von Willebrand syndrome is a rare bleeding disorder often secondary to an underlying lymphoproliferative disorder. We report a case in whom response of both the acquired von Willebrand syndrome and smoldering multiple myeloma persist 14 months after daratumumab treatment discontinuation.

Association between pacifier use and breast-feeding, sudden infant death syndrome, infection and dental malocclusion.

OBJECTIVE: To critically review all literature related to pacifier use for full-term healthy infants and young children.The specific review questions addressed are:What is the evidence of adverse and/or positive outcomes of pacifier use in infancy and childhood in relation to each of the following subtopics: INCLUSION CRITERIA: Specific criteria were used to determine which studies would be included in the review: (i) the types of participants; (ii) the types of research design; and (iii) the types of outcome measures. To be included a study has to meet all criteria.The participants included i

The Role of Platelet Dysfunctions in the Pathogenesis of the Hemostatic-Coagulant System Imbalances.

Platelet dysfunction plays a critical role in the pathogenesis of various disorders affecting the hemostatic-coagulant system. This review aims to explore the mechanisms by which platelet dysfunctions contribute to the disruption of hemostasis, leading to an increased risk of both thrombosis and bleeding. Platelets, traditionally known for their role in clot formation, can exhibit altered functionality under pathological conditions such as cardiovascular diseases, metabolic disorders, and autoimmune diseases, impacting their interaction with coagulation factors and vascular endothelium. The re

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… Desmopressin (DDAVP), which has been used for nearly 40 years to treat congenital bleeding disorders, works by increasing plasma levels of von Willebrand factor, factor VIII, and intracellular platelet calcium/sodium ion concentrations. … Immunate is a plasma-derived coagulation factor complex containing Factor VIII and von Willebrand factor. …

Thrombosis in multiple myeloma: risk stratification, antithrombotic prophylaxis, and management of acute events. A consensus-based position paper from an ad hoc expert panel.

The introduction of new therapeutic agents for multiple myeloma (MM), including proteasome inhibitors, immunomodulatory drugs, and monoclonal antibodies, has improved the outcomes of patients but, in parallel, has changed the frequency and epidemiology of thrombotic events. Thrombosis is now a significant cause of morbidity and mortality in MM patients, and optimal thromboprophylaxis is far from being reached. Moving from the recognition that the above issue represents an unmet clinical need, an expert panel assessed the scientific literature and composed a framework of recommendations for imp

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… It has been shown that serum levels of the anticoagulant cofactor thrombomodulin decrease in people treated with thalidomide. 66 Moreover, extremely high levels of von Willebrand factor antigen and factor VIII have been documented in people with MM receiving thalidomide, dexamethasone, and chemotherapy … Extremely high levels of von Willebrand factor antigen and of procoagulant factor VIII found in multiple myeloma patients are associated with activity status but not with thalidomide treatment. J Thromb Haemost. 2003;1(3):445-449. 68. …

Clinical characteristics and outcomes of IgD myeloma: experience across UK national trials.

Immunoglobulin D (IgD) myeloma is a subtype often considered to have adverse features and inferior survival, but there is a paucity of data from large clinical studies. We compare the clinical characteristics and outcomes of patients with IgD myeloma from UK phase 3 myeloma trials analyzed in 2 groups: old (1980-2002) and recent (2002-2016) clinical trials, based on the time of adoption of novel myeloma therapies. Patients with IgD myeloma comprised 44 of 2789 (1.6%) and 70 of 5773 (1.2%) of the old and recent trials, respectively. Overall, IgD myeloma was associated with male predominance, lo

Доклад по делу Ига-Ига-И типа Мильомы, совпадающей с Гиперлипидемией.

В большинстве случаев у пациентов с множественной миеломой (ММ) обычно наблюдаются нормальные или пониженные уровни липидов крови. Однако небольшое число случаев свидетельствует о значительной роли белка M в повышенных липидовых уровнях, особенно в тесной связи между типом IgA MM и гипертриглицеридемией и гиперхлестеролемией. Здесь мы сообщаем о случае MM типа Ига-Ига с одновременно гиперлипидемией, когда пациент с повышенным уровнем липидов при поступлении: полный холестерин 10.44 mmol/L (обычный диапазон: 2.8-5.6 mmol/L) и триглицериды 28.45 mmol/L (обычный диапазон: 0.45-1.70 mmol/L) Пациен

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… Further perfection through coagulation factor examination, immune‐related investigations, anemia assessment, and protein electrophoresis revealed a decrease in Factor VIII levels and coagulation factor deficiency (Table 2 ). … Project name | Result | Unit | Reference values FII:C% | 92 | % | 70–120 FV:C% | 33 | % | 70–120 FVII:C% | 238 | % | 55–170 FX:C% | 65 | % | 70–120 FVIII:C% | 4 | % | 70–150 Factor VIII antibody | 0 | BU | 0–0.6 FIX:C% | 51 | % | 60–150 FXI:C% | 50 | % | 70–120 FXII:C% | 75 | % | 60–150 PT | 14.2 | s …

Shortage of plasma-derived medicinal products: what is next? narrative literature review on its causes and counteracting policies in Italy.

Introduction: This paper describes the peculiarities of the plasma-derived medicinal product (PDMP) market and illustrates the results of a review of the literature on policies aimed at counteracting the shortage of PDMPs. Characteristics of PDMPs: Plasma is primarily used for the industrial production of blood products (80%). The demand for PDMPs, particularly immunoglobulins (IGs), is increasing. However, the production of PDMPs is complex, long (7-12 months), and expensive, accounting, according to US estimates, for 57% of the total costs of PDMPs compared to 14% for small molecules. PDMP m

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… The strategic and pivotal role of PDMPs has been confirmed by the inclusion of IGs and coagulation factors (VIII and IX) in the list of drugs considered essential for basic healthcare services by the World Health Organization ( World Health Organization, 2021 ). … In 2021, PDMPs produced via a self-sufficiency program in Italy covered 71% of the demand, ranging from 10% for subcutaneous IGs (82% for intravenous IGs) to 100% for Factor VIII ( Candura et al., 2023 ). …

Evidence for the use of intravenous immunoglobulins--a review of the literature.

Intravenous immunoglobulins (IVIg) were first introduced in the middle of the twentieth century for the treatment of primary immunodeficiencies. In 1981, Paul Imbach noticed an improvement of immune-mediated thrombocytopenia, in patients receiving IVIg for immunodeficiencies. This opened a new era for the treatment of autoimmune conditions with IVIg. Since then, IVIg has become an important treatment option in a wide spectrum of diseases, including autoimmune and acute inflammatory conditions, most of them off-label (not included in the US Food and Drug Administration recommendation). A panel

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… VIII). … with acquired factor VIII inhibitors 16 of 26 patients (62%) had inhibitors reduced in more than 25%; 7 of 26 patients (27%) had clinical benefit [ 93 ] Case series IVIg (Sandoglobulin) 2 g/kg over 5 days 4 patients with acquired factor VIII inhibitors 2 of 4 patients (50%) had inhibitors reduced in …